Pulmonary alveolar proteinosis
There are 3 categories of the condition:
- Autoimmune and hereditary PAP – Causes disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF).
- Congenital PAP – Genetic disorder of surfactant production; presents in neonates.
- Secondary PAP – Associated with mineral dust exposure, hematologic malignancies, and allogenic hematopoietic cell transplantation.
Signs and symptoms include dyspnea, particularly on exertion, cough, and low-grade fever. The classic radiographic pattern is "crazy paving" on high-resolution CT, but other patterns may be observed.
Neonates with congenital PAP present in the first 1-2 years of life with symptoms ranging from severe respiratory failure in the neonatal period to insidious chronic interstitial lung disease in later months.
J84.01 – Alveolar proteinosis
10501004 – Pulmonary alveolar proteinosis
- Infectious pneumonia – Pneumocystis pneumonia, nocardiosis, and Mycoplasma pneumonia, as well as other bacterial, viral, and fungal sources of pneumonia
- Lipoid pneumonia – from aspiration of lipoid material; basilar areas of ground glass opacities are more common
- Drug-related hypersensitivity reaction – consider with a history of exposure to amiodarone, bleomycin, methotrexate, or nitrofurantoin
- Acute interstitial pneumonia
- Cystic fibrosis
- Chronic obstructive pulmonary disorder / reactive airway disease
- Pulmonary embolism
- Congestive heart failure or pulmonary edema
Last Updated: 07/17/2019